Dr. Larry Schut isn’t the retiring type.
Oh, he tried it three years ago. But it didn’t take, and more than a year ago the Maple Lake resident rejoined the ataxia clinic he started at the University of Minnesota, with one purpose in mind: to find a cure for the disorder that ravaged generations of his family.
"They had one neurologist taking care of ataxia patients, and were desperately in need of help," he said in brushing off the idea of going back to work.
That elusive search continues today, which is why Schut – who turns 81 on Wednesday, Sept. 13 – is once again seeing patients suffering with ataxia.
It is also why he joined several others, all of whom were considerably younger, in setting out from the Clair Nelson Center in Finland Wednesday, Sept. 6, on the Superior Hiking Trail along the North Shore of Lake Superior. The hike is part of a fundraiser to heighten awareness of the condition, the Mind Over Miles 60 for 60 Hike to Cure Ataxia.
The NAF walk-a-thon is designed to call attention to the still-mostly-unheard-of neurological disorder, and also to raise funds for the foundation.
Due to rainy weather and slippery trails, he fell a couple of times and managed only about half of the 20 miles he anticipated to hike. But he plans to walk from St. Joseph’s to Melrose on the Lake Wobegon Trail this week, and when he’s done the miles will add up to 60. That is how many years ago since his father Henry and his uncle, Dr. John Schut, founded the National Ataxia Foundation in 1957.
"We’re walking one mile for each year," Larry Schut said.
The doctor has been a director of an Alzheimer’s Center at the VA in Minneapolis, and started a pain clinic for United Health in St. Paul. But largely his professional and his personal lives have meshed into one, with a concentrated focus on the same thing. Ataxia has ruled his life, and you will soon understand why.
Ataxia hits home
Ataxia is a degenerative disease of the nervous system, and afflicts people of all ages. It causes people to have little control over their muscles or voluntary movements, typically leading to a lack of coordination and balance, slurred speech, and difficulty walking, eating, swallowing and breathing. Symptoms and their progress vary widely, and the person becomes more disabled as the disease advances.
There are about 40 known types of ataxia, but in the Silver Creek and Maple Lake areas there was only one form of ataxia, and that was the one that afflicted the Schut family.
That particular form – spinocerebellar ataxia type 1 (SCA1) – was initially known as Schut’s disease, due to it being found exclusively within the families in this area.
It was also only the second instance of hereditary ataxia. However, in the other instance it skipped a generation, whereas in the Schut family it affected members of each generation.
Since 1915, 64 members of the Schut family have died from ataxia. As more came down with this inherited degenerative condition, they saw symptoms arrive earlier, usually in their 20s, and progress faster, resulting in death within 10-15 years.
That time frame prompted the title of a book Schut’s father wrote in 1978: "Ten Years to Live: One family’s struggle with a killer disease."
Henry was only 10 years old when he first saw his father struggling with chores on the farm. The condition was often first noticed in their lower limbs, and soon its victims began having difficulty keeping their balance while walking.
"My grandfather often got accused of drunken driving because of the way he staggered," Schut said.
Ataxia’s symptoms could easily be confused with drunkenness, and it wasn’t unusual for those symptoms to be misdiagnosed as either multiple sclerosis or ALS.
Henry’s father died at the age of 46 in 1924. He and his two brothers had 12 children; seven of the cousins contracted Schut’s ataxia.
Suddenly, many children were tasked with doing the chores on the farms, and were growing up in single-parent households. Neighbors and others in the surrounding area began to wonder if this deadly affliction was contagious.
It wasn’t, but with so much mystery surrounding what was happening to the family, speculation ran rampant.
It is now known that for many forms of ataxia, there can be several causes. In the Schuts’ case, it was an inherited defective gene that caused the condition. While Mayo Clinic doctors correctly diagnosed it as familial ataxia in 1915, they did not know what was causing it to run in this family, or have a cure.
Making it personal
Schut family members all have stories of when they first learned that relatives were in effect being given a death sentence. For Schut, he still vividly recalls an Easter dinner when he was perhaps no older than 5.
"My mother had made a nice ham dinner, and suddenly (aunt) Alice began to choke and turn blue," he said. It was left to his father to explain that his first cousin had this condition that was eventually going to be fatal.
Being surrounded by death from such an early age played a large role in these children’s lives. Schut’s father wanted to attend medical school, to study ataxia and help his family. But this was during the Great Depression, and instead he returned home to the farm in 1934 and raised a family.
However, Henry’s younger brother John also had a goal of finding a cure for what was decimating his family. He became a neurologist in the mid-1940s, and his comprehensive study of ataxia in his family was published in Archives of Neurology and Psychiatry in 1950.
Schut had that same drive to take on the curse that family members were still plagued with, and attended the University of Minnesota Medical College. His initial plans were to be a research scientist, but an immediate opportunity presented itself and instead he went to work at the Minneapolis Clinic of Neurology in Golden Valley, one of the largest neurology practices in the nation.
It was there in the 1970s when he first learned of two brothers in Sioux Falls, S.D., with a rare and incurable disorder. From that discovery, a dozen more people were diagnosed with SCA1. It led to the opening of several NAF-sponsored clinics, and gave Schut newfound hope of at least identifying what caused SCA1.
A process of elimination
Since Schut’s father had avoided the disorder, neither Schut or his brother and four sisters were at risk. But three of their father’s four siblings developed ataxia, so their children were all at risk.
Schut says that in 1970 he became dedicated to ridding ataxia from the family. At that time as many as 59 family members were still at-risk of developing the disease. He collected blood from relatives, both those with and without ataxia, and held clinics twice a year in Minneapolis.
Since those who contracted SCA1 were generally in their 20s – or about the same age that people began to get married and have children – if one of the Schuts were still symptom-free by the time they reached their early 30s, chances are they were safely in the clear.
Family members were advised to either delay having children until the time where they were past the greatest risk, avoid having children or to adopt.
Due to education about the dangers, only one family member currently has developed SCA1 and is in her final stages, Schut said, and one teenager is still at risk.
Making progress
Antibiotics and new medical methods kept Schut’s uncle, Dr. John, alive for longer than any other family member afflicted with ataxia. He lived with symptoms for 23 years before dying at age 51.
"He urged me to promise to keep him alive as long as possible, because he believed a cure was just around the corner," Schut said. "He was my model, and still is."
As research into SCA1 gained wider attention, more families who were affected by it in the U.S. were discovered. With additional cases to work with, medical advances followed.
Schut continued the genome association studies started by his uncle, and in 1984 he convinced Dr. Harry Orr to concentrate on trying to find the genetic cause of ataxia in the Schut family and another family in Michigan.
Nearly a decade later, Huda Zoghbi of the Baylor College of Medicine was doing the same thing with a Texas family, and rather than compete they agreed to collaborate. Their big breakthrough came in the spring of 1993, when they found what Schut refers to as "the real culprit," the mutant gene that causes SCA1.
It was the first dominantly-inherited ataxia gene discovered, and it was the result of an NAF grant to Orr and his research team at the University of Minnesota, which included Schut, who cloned the gene.
"I am honored to have been a small part of that team," he said.
That led at last to the creation of a genetic test to identify if someone carried the disorder.
"Since the gene was discovered in 1993, I have been more intent than ever on getting a cure," Schut said last week. "That’s why I am back at the university, and I promise to keep working on degenerative ataxia in general, and to keep seeing patients as long as I am able."
Although there are still no clinical-stage treatments for hereditary and sporadic ataxias, Schut said progress has been made in limiting the fatality of the disease in mice.
"I’m seriously hoping we’ll have trials in humans in two years," he said.
Turning up the spotlight
It’s hard to gauge exactly how many Americans have the various forms of ataxia, but reports put it in the neighborhood of 150,000.
"Hereditary or degenerative ataxias are as common as muscular dystrophy," Schut said. "We don’t have a spokesperson to give us exposure like Jerry Lewis, who raised more than $2 billion for MD."
Public awareness of ataxia remains limited. The National Ataxia Foundation, which now spends more than $1 million a year funding ataxia research in the U.S. and internationally, is committed to changing that.
That is why Schut and the others are walking 60 miles this month. His personal goal is to raise $10,000, and he is already about 40 percent of the way there. To contribute, send donations to: Larry Schut, 11091 County Road 7 NW, Maple Lake, MN 55358. Make checks out to the National Ataxia Foundation.
The foundation is holding several other events across the country, including a Walk N’ Roll to Cure Ataxia, such as the one this past weekend in St. Louis Park.
International Ataxia Awareness Day is Monday, Sept. 25, and is also geared to spread awareness about ataxia and inspire action towards a cure.
Schut’s uncle firmly believed a cure was around the corner. Significant progress has been made in the ensuing years, and research is that much closer to turning the corner.
Until that happens, Dr. Larry Schut doesn’t plan on slowing down at all.